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Swyer-James syndrome

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Known for heart disease, presented with dry cough and dyspnea.

Patient Data

Age: 35 years
Gender: Female
ct

The CT scan demonstrates:

  • decreased volume with hyperlucent left lung mainly the lower lobe with diminished vascularity, small pulmonary artery and its lobar and segmental branches, and cystic bronchiectases involving both, upper and lower lobes. Findings highly suggestive of Swyer-James syndrome
  • no filling defects are seen in the branches of the pulmonary arterial system
  • cardiomegaly with pericardial effusion, and left ventricular enlargement
  • mild left pleural effusion
  • moderate dilatation of the IVC and hepatic veins with contrast reflux from the right atrium into IVC (passive hepatic congestion)
  • emphysematous bullae of the right lung

Case Discussion

CT features most consistent with Swyer-James syndrome (SJS) involving the entire left lung. It is considered a rare lung condition that manifests as unilateral hemithorax lucency, usually the result of postinfectious obliterative bronchiolitis.

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